Anti-GCSH Antibody, Rabbit Polyclonal sinobiological 14568-RP01

$143.00
In stock
SKU
14568-RP01
Catalog No.SizePrice (USD)
14568-RP01-100100 µL$147.96
14568-RP01-200200 µL$251.64
14568-RP01-400400 µL$355.32

General Information

Product nameAnti-GCSH Antibody, Rabbit Polyclonal
Validated applicationsELISA (FAQ Protocol)
Species reactivityReacts with: Human
SpecificityHuman GCSH
ImmunogenRecombinant Human GCSH protein (Catalog#14568-H07E)
PreparationProduced in rabbits immunized with purified, recombinant Human GCSH (rh GCSH; Catalog#14568-H07E; AAA36011.1; Ser49-Glu173). Total IgG was purified by Protein A affinity chromatography.
SourcePolyclonal Rabbit IgG
PurificationProtein A
Formulation0.2 μm filtered solution in PBS
ConjugateUnconjugated
FormLiquid
ShippingThis antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
StorageThis antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles.

Synonyms: Anti-GCE Antibody; Anti-MMDS7 Antibody; Anti-NKH Antibody

Background Information

Full Nameglycine cleavage system protein H (aminomethyl carrier)
DescriptionDegradation of glycine is brought about by the glycine cleavage system, which is composed of four mitochondrial protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme), and L protein (a lipoamide dehydrogenase). GCSH is the H protein, which transfers the methylamine group of glycine from the P protein to the T protein. Defects in GCSH gene are a cause of nonketotic hyperglycinemia (NKH). Two transcript variants, one protein-coding and the other probably not protein-coding,have been found for GCSH gene. Also, several transcribed and non-transcribed pseudogenes of GCSH gene exist throughout the genome.
References
  1. Hiraga K. et al., 1988, Biochem Biophys Res Commun. 151 (2): 758-62.
  2. Fujiwara K. et al., 1991, Biochem Biophys Res Commun. 176 (2): 711-6.
  3. Koyata H. et al., 1991, Am J Hum Genet. 48 (2): 351-61.

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